Long-term prognosis of patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

Qian, Jianfei; Li, Mengtao; Zhang, Xiao; Wang, Qian; Tian, Zhuang; Wei, Wei; Zuo, Xiaoxia; Zhang, Miaojia; Zhu, Ping; Ye, Shuang; Zhang, Wei; Zheng, Yi; Qi, Wufang; Yang, Li; Zhang, Zhuoli; Ding, Feng; J, Gu; Liu, Yi; Wang, Yanhong; Zeng, Xiaofeng

doi:10.1183/13993003.00081-2018

Cited by 58 publications

(75 citation statements)

References 32 publications

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“…A T2T strategy in SLE, which asserted that complete remission is the main target of SLE therapy, has been recommended by an international task force . Qian et al had already reported that treatment goal achievement (TGA) was associated with long‐term survival, which supports the T2T strategy in SLE‐associated PAH. However, challenges are met in the subgroup with pSS‐PAH.…”

Section: Discussionmentioning

confidence: 99%

Characteristics and risk factors for pulmonary arterial hypertension associated with primary Sjögren’s syndrome: 15 new cases from a single center

et al. 2019

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Aim This study aimed to retrospectively describe 15 new primary Sjögren's syndrome‐pulmonary arterial hypertension (pSS‐PAH) cases confirmed by right heart catheterization (RHC). Demographic and clinical characteristics were analyzed and risk factors for PAH in pSS were explored. Method We retrospectively described 15 new pSS‐PAH cases confirmed by RHC referred to our institution between January 2013 and March 2018. We present PAH and pSS characteristics, hemodynamic evaluations, medical management, and outcomes. A matched case control study was carried out to determine the risk factors of PAH in pSS compared with pSS‐non‐PAH patients. Results All patients were female with a mean age at PAH diagnosis of 52.9 ± 14.6 years. The delay between the first symptom and PAH diagnosis was 18.7 ± 19.7 months. The most common primary manifestation at PAH onset was exertional dyspnea (13/15). At diagnosis of PAH, PAH was severe with a mean pulmonary artery pressure of 48.8 ± 13.7 mm Hg (range, 27‐72 mm Hg) and a mean cardiac index of 2.3 ± 0.6 L/min/m2 (range, 1.47‐3.41 L/min/m2). Compared with the pSS‐PAH without pericardial effusion, pSS‐PAH with pericardial effusion had larger right arterial (53 [45‐56.75] vs 38 [35.5‐46.5], P = .018) and right ventricular sizes (47 [42.75‐51.25] vs 36 [32.5‐41], P = .007). Compared with the pSS non‐PAH group, we identified 2 risk factors for PAH in pSS: pericardial effusion (odds ratio [OR] [95% CI], 14.29 [1.14‐166.67], P = .039) and liver involvement (OR [95% CI], 14.71 [1.14‐166.67], P = .035). Conclusion For pSS patients, PAH can be the first manifestation. We believe that systemic evaluation, especially in patients with pericardial effusion and liver involvement, is important to identify high‐risk patients for PAH, improving their prognosis.

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Section: Discussionmentioning

confidence: 99%

Characteristics and risk factors for pulmonary arterial hypertension associated with primary Sjögren’s syndrome: 15 new cases from a single center

et al. 2019

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“…Although there have been a number of survival studies of SLE in the past decade, not too many were performed in the Asian populations (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25). Analysis of the survival of hospitalized patients (9,22), those admitted to the intensive care unit (12,19) or referred to tertiary centers (23), subsets of patients with nephritis (14), neuropsychiatric manifestations (17), or pulmonary hypertension (15) in some studies would create bias in the mortality rate. Moreover, the follow-up of most of these Asian studies was not long enough to look at the trend of survival over time.…”

Section: Introductionmentioning

confidence: 99%

Trend of Survival of a Cohort of Chinese Patients With Systemic Lupus Erythematosus Over 25 Years

Mok

Chan

et al. 2020

Front. Med.

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Objectives: To revisit the trend of survival of systemic lupus erythematosus in a cohort of Chinese patients over 25 years. Methods: Patients who fulfilled the 1997 ACR criteria for SLE and were followed in our hospital since 1995 were included. Patients were stratified into two groups according to the year of diagnosis: (1) 1995-2004 and (2) 2005-2018. Survival of patients was studied by Kaplan-Meier analysis. Organ damage as assessed by the Systemic Lupus International Collaborating Clinics (SLICC) damage index (SDI) and causes of death in the first 10 years of SLE onset was compared between the two groups. Cox regression was used to study factors associated with survival. Results: A total of 1,098 SLE patients were registered in our database. After excluding 157 patients diagnosed outside the time period of 1995-2018, 941 patients were studied (92% women). All were ethnic Chinese. The mean age of SLE onset was 35.1 ± 14.4 years, and the mean duration of observation was 13.1 ± 6.6 years. Seventy-seven (8.2%) patients were lost to follow-up. Groups 1 and 2 consisted of 364 and 577 patients, respectively. The mean SDI score at 10 years of disease onset was significantly higher in group 1 than group 2 patients (1.01 ± 1.43 vs. 0.57 ± 0.94; p < 0.01), particularly in the neuropsychiatric, musculoskeletal, and gonadal domains. Within 10 years of SLE onset, 32 (8.8%) patients in group 1 and 25 (4.3%) patients in group 2 died (p = 0.005). The 5-and 10-year cumulative survival rates were 93.6 and 91.0% in group 1 and 96.5 and 94.2% in group 2 patients, respectively (log-rank test p = 0.048). Infection accounted for more than half of the deaths in both groups. More group 1 than group 2 patients died of vascular events, but the difference was not statistically significant. Cox regression showed that the age of SLE onset and damage score accrued at 10 years, but not the time period in which SLE was diagnosed, were significantly associated with mortality. Conclusions: The improvement in survival of our SLE patients is probably related to the accrual of less organ damage in the past 15 years.

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“…All of the selected patients fulfilled the following inclusion criteria: ‹ age !18 years; › intensive immunosuppressive therapy initiated at our hospital; and fi regular follow-up. Intensive immunosuppressive therapy was defined as therapy with glucocorticoids and immunosuppressants [18]. Regular follow-up means that patients visited specialist physicians regularly every 1-3 months.…”

Section: Methodsmentioning

confidence: 99%

Infection-related hospitalization after intensive immunosuppressive therapy among lupus nephritis and ANCA glomerulonephritis patients

Yin

Wen

et al. 2020

Renal Failure

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Introduction: This study aimed to investigate the clinical characteristics, risk factors, and outcomes of infection-related hospitalization (IRH) in patients with lupus nephritis (LN) and ANCA glomerulonephritis after intensive immunosuppressive therapy. Methods: Patients diagnosed with LN or ANCA glomerulonephritis who received intensive immunosuppressive therapy at the First Affiliated Hospital of Sun Yat-sen University from 2005 to 2014 were enrolled. Demographics, laboratory parameters, immunosuppressive agents, and IRH details were collected. Multivariable Cox regression was used, and hazard ratios (HRs) and 95% confidence intervals (CIs) were reported. Results: Totally, 872 patients with 806 LN and 66 ANCA glomerulonephritis were enrolled, and 304 (34.9%) patients with 433 episodes of IRH were recorded. ANCA glomerulonephritis patients were more vulnerable to IRH than LN patients (53.0% vs. 33.4%, p ¼ .001). Multivariable Cox regression analysis showed that ANCA glomerulonephritis (HR ¼ 1.62, 95% CI: 1.06-2.49, p ¼ .027), diabetes (HR ¼ 1.82, 95% CI: 1.03-3.22, p ¼ .039) and a higher initial dose of prednisone (HR ¼ 1.01, 95% CI: 1.00-1.02, p ¼ .013) were associated with a higher likelihood of IRH. Higher albumin (HR ¼ 0.96, 95% CI: 0.94-0.98, p < .001), globulin (HR ¼ 0.98, 95% CI: 0.96-0.99, p ¼ .008), and eGFR (HR ¼ 0.99, 95% CI: 0.99-1.00, p < .001), were associated with a lower likelihood of IRH. The rates of transfer to ICU and mortality for ANCA glomerulonephritis patients were higher than those for LN patients (22.9% vs. 1.9%, p < .001, and 20.0% vs. 0.7%, p < .001, respectively). Conclusions: ANCA glomerulonephritis patients had a higher risk of IRH and poorer outcome once infected after intensive immunosuppressive therapy than LN patients. More strict control for infection risks is required for ANCA glomerulonephritis patients who undergo intensive immunosuppressive therapy.

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Long-term prognosis of patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study

Cited by 58 publications

References 32 publications

Characteristics and risk factors for pulmonary arterial hypertension associated with primary Sjögren’s syndrome: 15 new cases from a single center

Characteristics and risk factors for pulmonary arterial hypertension associated with primary Sjögren’s syndrome: 15 new cases from a single center

Trend of Survival of a Cohort of Chinese Patients With Systemic Lupus Erythematosus Over 25 Years

Infection-related hospitalization after intensive immunosuppressive therapy among lupus nephritis and ANCA glomerulonephritis patients

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